Introduction: The treatment of children with rhabdomyosarcoma (RMS) ha
s been established in multicenter protocols. There are few data availa
ble regarding the treatment of other sarcomas in pediatric patients. M
ethods: From 1955 to 1988, a retrospective review of 33 children with
sarcomas other than RMS of the head and neck was performed to identify
prognostic factors and to determine optimal treatment. Grade, tumor s
ize, and RMS grouping of the tumors were found to be of prognostic val
ue. Results: Eighteen of 33 (54%) patients are currently disease free.
Only 3 of 16 (19%) patients treated initially with surgery alone were
rendered disease free. However, 6 patients were salvaged with an addi
tional surgical procedure, yielding 9 of 16 (56%) patients disease fre
e treated with surgery alone. Of patients receiving surgery plus radia
tion therapy, with or without chemotherapy, 7 of 14 (50%) have no evid
ence of disease. Conclusion: After salvage therapy, 5-year disease-fre
e survival was 56% (15 of 27). Unlike rhabdomyosarcoma, complete surgi
cal extirpation is the mainstay of treatment in pediatric sarcomas oth
er than RMS of the head and neck. Adjuvant radiation with or without c
hemotherapy should be administered when complete resection is not poss
ible. Copyright (C) 1995 by W.B. Saunders Company